Giant intra-cardiac undifferentiated pleomorphic sarcoma: a case report

Abstract Background Primary cardiac tumours are rare. Though most are benign, they significantly interfere with cardiac function. Malignant tumours, predominantly sarcomas, include the highly aggressive undifferentiated pleomorphic cardiac sarcoma that often manifests with non-specific symptoms. Case Summary A 72-year-old man presented with weeks-long fatigue, appetite loss, and chronic dyspnea (NYHA III) following a previous acute coronary syndrome and occupational exposure to toxic solvents. His examination revealed dyspnea and elevated blood pressure, while imaging uncovered a large intra-cavitary mass at the junction of the inferior vena cava and right atrium. Multiple metastatic lesions to the pleura, lung, pericardium and lymph nodes were identified on CT, PET-CT, and MRI, with ultrasound confirming significant tricuspid stenosis caused by the mass. Histopathological analysis of a pleural metastasis established the diagnosis of an undifferentiated pleomorphic sarcoma. Discussion Comprehensive imaging modalities (CT, PET-CT, MRI, ultrasound) are essential for characterizing cardiac masses and staging malignancy. Although surgical resection is the primary treatment, palliative chemotherapy with agents such as pazopanib, carboplatin, gemcitabine, and docetaxel is employed due to widespread metastases and poor prognosis. Undifferentiated pleomorphic cardiac sarcomas remain diagnostically challenging, with treatment focused on symptom relief and palliative care in advanced disease. Early detection remains vital for quality of life.